Fuchs endothelial corneal dystrophy.

Fuchs endothelial corneal dystrophy.
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DOI:
10.1016/s1542-0124(12)70232-x
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发表时间:
2010-10
期刊:
影响因子:
6.4
通讯作者:
Jurkunas, Ula V.
Jurkunas, Ula V.
中科院分区:
医学2区
文献类型:
--
作者:
Elhalis, Hussain;Azizi, Behrooz;Jurkunas, Ula V.

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Fuchs角膜内皮营养不良(FECD)的特征在于角膜内皮细胞的进行性丧失、Descement膜的增厚和细胞外基质以滴状的形式沉积。当内皮细胞的数量变得非常低时,角膜肿胀并导致视力丧失。FECD的临床过程通常跨越10-20年。角膜移植是目前唯一用于恢复视力的方法。在过去的几十年里,遗传学研究已经发现了几个基因,以及与疾病相关的染色体位点区域。蛋白质组学的研究已经引起了几个假说的发病机制FECD。这篇综述扩展了蛋白质组学和遗传学研究的最新发现,并建立在最近的进展,了解这种常见的角膜疾病的原因。
Fuchs endothelial corneal dystrophy (FECD) is characterized by progressive loss of corneal endothelial cells, thickening of Descement’s membrane and deposition of extracellular matrix in the form of guttae. When the number of endothelial cells becomes critically low, the cornea swells and causes loss of vision. The clinical course of FECD usually spans 10–20 years. Corneal transplantation is currently the only modality used to restore vision. Over the last several decades genetic studies have detected several genes, as well as areas of chromosomal loci associated with the disease. Proteomic studies have given rise to several hypotheses regarding the pathogenesis of FECD. This review expands upon the recent findings from proteomic and genetic studies and builds upon recent advances in understanding the causes of this common corneal disorder.
DOI: 10.1074/jbc.m412569200
发表时间: 2005-04-08
影响因子: 4.8
作者:
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